Nutritional counseling improves quality of life and preserves body weight in systemic immunoglobulin light-chain (AL) amyloidosis

Nutrition ◽  
2015 ◽  
Vol 31 (10) ◽  
pp. 1228-1234 ◽  
Author(s):  
Riccardo Caccialanza ◽  
Giovanni Palladini ◽  
Emanuele Cereda ◽  
Chiara Bonardi ◽  
Paolo Milani ◽  
...  
2021 ◽  
Author(s):  
Shana Souza Grigoletti ◽  
Priccila Zuchinali ◽  
Emilie Lemieux-Blanchard ◽  
Stephanie Bechard ◽  
Bernard Lemieux ◽  
...  

Background: Immunoglobulin light chain (AL) amyloidosis is a complex disease marked by a poor clinical portrait and prognosis generally leading to organ dysfunction and shortened survival. We aimed to review the available evidence on whether AL amyloidosis can lead to malnutrition, thus having a negative impact on quality of life (QoL) and survival. Materials: We searched Pubmed with no restrictions to the year of publication or language. Retrospective or prospective, observational, and interventional studies that reported data regarding AL amyloidosis and nutritional status were included. Results: From 62 articles retrieved, 23 were included. Malnutrition was prevalent in up to 65% of patients with AL Amyloidosis. Prevalence of weight loss of 10% or more ranged from 6 to 22% of patients, while a body mass index of < 22 kg/m2 was found in 22 to 42%. Weight loss, lower BMI and other indicators of poor nutritional status were negatively associated with quality of life and survival. Only one RCT focused on nutritional counselling was found and reported positive results on patients' QoL and survival. Conclusion Despite inconsistencies across assessment criteria, the available data reveal that weight loss and malnutrition are common features in patients with AL amyloidosis. This review reinforces the premise that an impaired nutritional status can be negatively associated with QoL and survival in patients with AL amyloidosis, and therefore should be further investigated.


2011 ◽  
Vol 91 (3) ◽  
pp. 399-406 ◽  
Author(s):  
Riccardo Caccialanza ◽  
Giovanni Palladini ◽  
Catherine Klersy ◽  
Emanuele Cereda ◽  
Chiara Bonardi ◽  
...  

2019 ◽  
Vol 9 (6) ◽  
Author(s):  
Victoria E Thomas ◽  
Justin Smith ◽  
Merrill D Benson ◽  
Noel R Dasgupta

Amyloidosis is a group of diseases characterized by extracellular deposition of amyloid fibril complexes. Fibril deposition results in organ dysfunction and possible failure. Amyloidosis is regarded as a rare disease, but in general is underdiagnosed. The two main types of systemic amyloidosis are immunoglobulin light chain and transthyretin amyloidosis. The increased availability of noninvasive cardiac imaging, genetic testing and improved laboratory assays and protein identification methods have led to increased diagnosis. However, in many cases, the diagnosis is not made until the patient develops organ impairment. Earlier diagnosis is required to prevent irreversible organ failure. Novel treatments for immunoglobulin light chain and transthyretin amyloidosis that halt disease progression, prolong and increase quality of life have recently become available.


2021 ◽  
Vol 14 (4) ◽  
pp. e240631
Author(s):  
Ethan Mar ◽  
Kerry Taylor ◽  
Peter Mollee

We report a case of progressive light-chain amyloidosis (otherwise known as AL amyloidosis) with acquired factor X (aFX) deficiency with a complete haematological response and rapid normalisation of FX levels following daratumumab monotherapy. To our knowledge, this is the first case report documenting successful treatment with daratumumab of aFX deficiency secondary to AL amyloidosis. The patient responded well to this therapy, with excellent symptomatic and quality of life improvements as well as a reduction in bleeding manifestations. This case highlights the value in considering daratumumab treatment when AL amyloidosis is complicated by FX deficiency.


2021 ◽  
Vol 8 ◽  
Author(s):  
Wei Yan ◽  
Peng Li ◽  
Cen Wu ◽  
Chuming Zhou ◽  
Aijun Liao ◽  
...  

Primary tracheobronchial light chain (AL) amyloidosis is a rare and heterogeneous disease characterized by the buildup of amyloid deposits in the airway mucosa. Although its treatment remains challenging, the current view is that the localized form can be treated conservatively due to its slow progression. While radiotherapy has proven effective in treating localized form of the disease, some patients do not respond to local treatment and continue to experience poor quality of life, highlighting the need to explore additional treatment strategies. In this report, we discuss a case of primary tracheobronchial AL amyloidosis with biclonal gammopathy (IgA κ and IgG κ) in a 46-year-old man who was transferred to our hospital due to dyspnea progression over the preceding 3 years. Chest computed tomography revealed irregular tracheobronchial stenosis with wall thickening, and histological examination of the bronchial biopsies confirmed the diagnosis of endobronchial AL amyloidosis. Owing to the poor effect of radiation therapy and treatments for improving airway patency, he was treated with a systemic chemotherapy regimen [cyclophosphamide-bortezomib-dexamethasone (CyBorD)]. We observed substantial improvements in his dyspnea, highlighting the potential of systemic therapy to improve quality of life of patients with tracheobronchial AL amyloidosis. However, the long-term pathological changes associated with local bronchial lesions require further investigation.


1993 ◽  
Vol 11 (10) ◽  
pp. 2043-2049 ◽  
Author(s):  
L Ovesen ◽  
L Allingstrup ◽  
J Hannibal ◽  
E L Mortensen ◽  
O P Hansen

PURPOSE This study examined the effect of frequent nutritional counseling on oral intake, body weight, response rate, survival, and quality of life in patients with cancer of the lung (small-cell), ovary, or breast undergoing cyclic chemotherapy. PATIENTS AND METHODS Of 105 assessable patients, 57 were randomized to receive nutritional counseling, and 48 to receive no nutritional counseling and consumption of an ad lib oral intake. The intervention group was counseled to achieve a daily energy and protein intake according to recommended dietary allowances. Counseling was standardized and performed by a trained dietitian, and took place twice monthly during a 5-month period from start of chemotherapy. RESULTS Dietary counseling increased daily energy intake by approximately 1 MJ and protein intake by 10 g over the entire study period. There was no change in the control group. Counseling led to an insignificant increase in body weight, but triceps skinfold measurement increased significantly after 5 months. Response rate and overall survival did not differ between the groups. Quality of life measured by the Quality-of-Life index (QL-index) increased significantly in both groups, but did not differ between groups. CONCLUSION No clinical benefit could be demonstrated despite long-term and continuous improved food intake in cancer patients with solid tumors undergoing aggressive chemotherapy.


2014 ◽  
Vol 32 (31_suppl) ◽  
pp. 67-67
Author(s):  
Neha Kapoor ◽  
Ihab Tewfik ◽  
Jane Naufahu ◽  
Sundus Tewfik ◽  
Rakesh Garg ◽  
...  

67 Background: The prevalence of malnutrition, amongst cancer palliative patients, is 81%. As cancer progresses, patients’ develop malnutrition owing to metabolic changes and ill-effects of treatment. The aim of this study was to improve nutritional status of female cancer patients and their quality of life by counseling and providing nutrient rich natural food (IAtta). Methods: Female cancer patients (n=45) attending palliative care clinic (AIIMS, New Delhi); with symptoms of cachexia were randomly distributed into control (Group one, n=23) and intervention group (Group two, n=22). Both groups were provided with nutritional counseling while the intervention group also received 100grams of IAtta, to be consumed daily for three months. Anthropometric measurements [weight, muscle mass, percentage body fat (%BF) and mid upper arm circumference (MUAC)], dietary intake (two day dietary recall) and quality of life (European Organisation for Research and Treatment of Cancer-Quality of Life C30 Questionnaire) were assessed at baseline and after three months. Data was analysed using paired t-test and Wilcoxon signed-rank test on the variables assessed. P-value < 0.05 was considered statistically significant at 95% confidence interval. Results: Patients in group one had significantly decreased body weight (p= 0.001), muscle mass (p= 0.021), MUAC (p= 0.005) and %BF (p= 0.001) by end of three months. While patients in group two reported significant improvement in fatigue (p= 0.001) and appetite loss score (p= 0.002) under quality of life domains. Conclusions: Nutritional counseling along with IAtta food supplementation helps in stabilising weight and improves quality of life of cancer cachexia patients undergoing palliative care treatment. Clinical trial information: RP- 02/17.06.2013. [Table: see text]


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 10098-10098 ◽  
Author(s):  
Paolo Pedrazzoli ◽  
Emanuele Cereda ◽  
Silvia Cappello ◽  
Sara Colombo ◽  
Catherine Klersy ◽  
...  

10098 Background: The benefit of systematic use of oral nutritional supplements (ONS) in addition to nutritional counseling in head and neck cancer (HNC) patients undergoing radiotherapy (RT) has not still been properly assessed. Methods: In a single-center, randomized, pragmatic, parallel-group controlled trial (ClinicalTrials.gov: NCT02055833; February 2014 - August 2016), 159 newly diagnosed HNC patients suitable for to RT regardless of previous surgery and induction chemotherapy were randomly assigned to nutritional counseling in combination with omega-3 enriched ONS (N = 78) or without ONS (N = 81) from the start of RT and continuing for up to 3 months after its end. The primary endpoint was the change in body weight at the end of RT. Secondary endpoints included changes in protein-calorie intake, muscle strength, body composition and quality of life (EORTC-QLQ-C30) over the study time points and anti-cancer treatment tolerance. Results: In patients in whom all the variables could be assessed, counseling plus ONS (N = 67) resulted in smaller loss of body weight than nutritional counseling alone (N = 69; mean difference, 1.6 kg [95%CI, 0.5 to 2.7]; P = 0.006). Imputation of missing outcomes provided consistent findings. In the ONS-supplemented group, higher protein-calorie intake and improvement in quality of life over time were also observed (P < 0.001 for all). The use of ONS reduced the need for changes in scheduled anti-cancer treatments (i.e. for RT and/or systemic treatment dose reduction or complete suspension, HR = 0.40 [95%CI, 0.18 to 0.91], P = 0.029). Nine patients reported gastrointestinal intolerance to ONS. Conclusions: In HNC patients undergoing RT or RT plus systemic treatment, and receiving nutritional counseling, weight loss could not be completely prevented, but the use of ONS resulted in better weight maintenance, increased protein-calorie intake, improved quality of life and better anti-cancer treatment tolerance. Clinical trial information: NCT02055833.


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